Awaji criteria improves the diagnostic sensitivity in amyotrophic lateral sclerosis: A systematic review using individual patient data

•Individual patient data analysis disclosed a higher sensitivity of the Awaji criteria when compared to the revised El Escorial criteria.•Modification of Awaji criteria, to include a “possible” diagnostic category significantly enhanced the diagnosis of ALS, particularly in limb onset disease.•Disease duration exerted a significant effect on the diagnostic utility of the Awaji criteria.

ObjectiveTo determine the utility of the Awaji criteria in diagnosing amyotrophic lateral sclerosis (ALS) and to propose a novel modification so as to enhance sensitivity based on results of individual patient data (IPD).MethodsIndividual patient data were available from 8 studies comparing the diagnostic accuracy of Awaji and revised El Escorial (rEEC) criteria. The sensitivity of a novel updated Awaji criteria, incorporating a “probable-laboratory supported” category, was also tested.ResultsIndividual patient data were available from 1086 patients, consisting of 881 ALS and 205 patients with disorders mimicking ALS. Summary sensitivities based on random effects logistic regression modelling disclosed a higher sensitivity of the Awaji criteria (0.70, 95% confidence interval [CI] 0.51-0.83) and updated Awaji criteria (0.73, 95% CI 0.56-0.85) when compared to rEEC (0.58, 95% CI 0.48-0.68). Paired analysis revealed higher sensitivities of Awaji criteria in 4 studies, and of updated Awaji criteria in 7 studies, when compared to rEEC.ConclusionIndividual patient data analysis established a higher sensitivity of Awaji criteria when compared to rEEC. The updated Awaji criteria enhanced the diagnostic sensitivity in limb-onset ALS.SignificanceThe updated Awaji criteria should be considered in clinical practice and future therapeutic trials.